Surveillance Guidelines
Primary Adrenal Insufficiency
If mineralocorticoid production is sufficient at the time of initial diagnosis, long-term follow up of adrenal mineralocorticoid function (sodium, potassium, aldosterone, plasma renin activity) is necessary. Monitoring should be fairly intense in the first two years of life (e.g., every 4 months) or at times of clinical concern. With age, mineralocorticoid sensitivity improves, but annual reviews would be appropriate and care needed during times of limited salt intake, fluid restriction, fluid loss (e.g., vomiting, diarrhea), or extreme heat. Clinical concern in the older child (e.g., postural hypotension or dizziness) needs investigation. If glucocorticoid production is sufficient at the time of initial diagnosis, long-term follow up of adrenal glucocorticoid function (basal ACTH, cortisol, cosyntropin test) is necessary. Basal ACTH is a useful marker of impaired glucocorticoid function and should be measured together with cortisol during the first two years of life. If there are any concerns, a cosyntropin stimulation test should be performed, looking for an impaired cortisol response, not just an inadequate basal cortisol level. Annual reviews of basal ACTH/cortisol and possibly cosyntropin stimulation should be considered in a boy with a genuine X-linked AHC if glucocorticoid insufficiency has not yet developed. Any clinical concerns (e.g.
